Tuccari G, Giuffrè G, Crisafulli C, Monici M C, Toscano A, Vita G
Department of Human Pathology, Policlinico Universitario, Messina, Italy.
Acta Neuropathol. 1999 Mar;97(3):247-52. doi: 10.1007/s004010050981.
We have investigated the quantity of argyrophilic nucleolar organizer region (AgNOR) proteins in vastus lateralis muscle samples from 13 patients with Duchenne muscular dystrophy (DMD) (6 months-12 years), 9 with Becker muscular dystrophy (BMD) (13 months-36 years), 9 with polymyositis (PM) (8-77 years) and 10 normal subjects (5 months-32 years). AgNORs were visualized on 4-microm-thick cryostat sections and quantified according to the guidelines of the Committee on AgNOR Quantitation; statistical analysis was performed on the mean AgNOR area (NORA) values. The mean NORA values encountered in DMD (4.327+/-0.791 microm2), BMD (3.534+/-0.312 microm2) and PM (3.785+/-0.424 microm2) samples were significantly (P<0.001) higher than those of normal muscle (1.682+/-0.288 microm2); a value of P<0.001 was also obtained when NORA values found in DMD were compared with those of BMD or PM. In addition, when NORA values were exclusively calculated in regenerating myofibers in DMD, BMD and PM, no differences were appreciable. On the other hand, in non-regenerating myofibers, the NORA values showed a significant increase in DMD versus BMD and PM (P<0.001) as well as in each disease group versus controls. Our study documents that muscle diseases, such as DMD, BMD and PM in which regeneration is a constant finding, have a high rDNA transcriptional activity. In particular, our findings suggest that (1) regenerating nuclei behave in the same way in dystrophinopathies or PM; (2) virtually all nuclei, including quiescent-looking ones, are activated to realize an increased intracellular protein synthesis for proliferative and/or functional purposes; and (3) the quantity of AgNOR does not seem related to age of patients at the time of biopsy.
我们研究了13例杜氏肌营养不良症（DMD）（6个月至12岁）、9例贝克肌营养不良症（BMD）（13个月至36岁）、9例多发性肌炎（PM）（8至77岁）患者以及10名正常受试者（5个月至32岁）的股外侧肌样本中嗜银核仁组织区（AgNOR）蛋白的数量。在4微米厚的低温恒温器切片上观察AgNOR，并根据AgNOR定量委员会的指导方针进行定量；对平均AgNOR面积（NORA）值进行统计分析。DMD（4.327±0.791平方微米）、BMD（3.534±0.312平方微米）和PM（3.785±0.424平方微米）样本中的平均NORA值显著高于正常肌肉（1.682±0.288平方微米）（P<0.001）；将DMD中发现的NORA值与BMD或PM的NORA值进行比较时，P值也<0.001。此外，当仅计算DMD、BMD和PM中再生肌纤维的NORA值时，没有明显差异。另一方面，在非再生肌纤维中，DMD与BMD和PM相比，NORA值显著增加（P<0.001），并且每个疾病组与对照组相比也是如此。我们的研究表明，在DMD、BMD和PM等肌肉疾病中，再生是一个常见现象，具有较高的核糖体DNA转录活性。特别是，我们的研究结果表明：（1）在肌营养不良症或PM中，再生核的行为方式相同；（2）几乎所有的核，包括看似静止的核，都会被激活，以实现细胞内蛋白质合成的增加，用于增殖和/或功能目的；（3）AgNOR的数量似乎与活检时患者的年龄无关。
